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2. Effect of Immunosuppressive Treatments on Kidney Outcomes After Gross Hematuria-Related Acute Kidney Injury in Older Patients With IgA Nephropathy

Author

Sevillano, Angel M., Caravaca-Fontán, Fernando, Garcia-Galan, Lucia Cordero, Fernandez-Juarez, Gema, Lopez-Revuelta, Katia, Guzmán, Diomaris A., Martín-Reyes, Guillermo, Quintana, Luis F., Rodas, Lida M., Sanchez de la Nieta, Maria Dolores, Rabasco, Cristina, Espinosa, Mario, Diaz-Encarnación, Monserrat, San Miguel, Luz, Barrios, Clara, Rodriguez, Eva, Garcia, Patricia, Valera, Alfonso, Peña, Jessy-Korina, Shabaka, Amir, Velo, Mercedes, Sierra, Milagros, Gonzalez, Fayna, Fernandez-Reyes, Maria José, Heras, Manuel, Delgado, Patricia, Gutierrez, Eduardo, Moreno, Juan Antonio, Praga, Manuel, and Cordero Garcia-Galan, Lucia

Published
2023
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3. Management of immunosuppressive therapy in kidney transplant recipients with COVID-19. A multicentre national study derived from the Spanish Society of Nephrology COVID registry

Author

López-Oliva, María O., Pérez-Flores, Isabel, Molina, María, José Aladrén, María, Trujillo, Hernando, Redondo-Pachón, Dolores, López, Verónica, Facundo, Carme, Villanego, Florentino, Rodríguez, Marisa, Carmen Ruiz, Maria, Antón, Paula, Rivas-Oural, Alba, Cabello, Sheila, Portolés, José, de la Vara, Lourdes, Tabernero, Guadalupe, Valero, Rosalía, Galeano, Cristina, Moral, Esperanza, Ventura, Ana, Coca, Armando, Ángel Muñoz, Miguel, Hernández-Gallego, Román, Shabaka, Amir, Ledesma, Gabriel, Bouarich, Hanane, Ángeles Rodríguez, María, Pérez Tamajón, Lourdes, Cruzado, Leónidas, Emilio Sánchez, José, and Jiménez, Carlos

Published
2023
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4. Manejo de la inmunosupresión en pacientes trasplantados de riñón con COVID19. Estudio multicéntrico nacional derivado del registro COVID de la Sociedad Española de Nefrología

Author

López-Oliva, María O., Pérez-Flores, Isabel, Molina, María, Aladrén, M. José, Trujillo, Hernando, Redondo-Pachón, Dolores, López, Verónica, Facundo, Carme, Villanego, Florentino, Rodríguez, Marisa, Ruiz, M. Carmen, Antón, Paula, Rivas-Oural, Alba, Cabello, Sheila, Portolés, José, de la Vara, Lourdes, Tabernero, Guadalupe, Valero, Rosalía, Galeano, Cristina, Moral, Esperanza, Ventura, Ana, Coca, Armando, Muñoz, Miguel Ángel, Hernández-Gallego, Román, Shabaka, Amir, Ledesma, Gabriel, Bouarich, Hanane, Rodríguez, M. Ángeles, Pérez Tamajón, Lourdes, Cruzado, Leónidas, Sánchez, J. Emilio, and Jiménez, Carlos

Published
2023
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5. #2903 Comprehensive lipidomic analysis reveals further insights into the molecular mechanism of podocyte lipotoxicity in Alport syndrome

Author

Shabaka, Amir, primary, Requena, Belen Fernandez, additional, Gonzalez-Riano, Carolina, additional, Lanzon, Borja, additional, Martinez, Sara, additional, Gradillas, Ana, additional, Carrillo, Isabel Galan, additional, Bosch, Teresa Bada, additional, Sevillano, Angel, additional, Medina-Gomez, Gema, additional, Barbas, Coral, additional, and Juarez, Gema Maria Fernandez, additional

Published
2024
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15. #3073 Should we expand the indications for hemodialysis in lithium intoxications beyond the EXTRIP recommendations?

Author

Shabaka, Amir, primary, Barriopedro, Mario Botella, additional, Triska, Eugenia Landaluce, additional, Pérez, Javier Vian, additional, Ortega, María José, additional, Belotto, Miguel Martínez, additional, Procaccini, Fabio, additional, Menendez, Veronica Rubio, additional, Mendoza, Ana, additional, Sanz, Juan Manuel Acedo, additional, De Pedro, Maria Patricia Sanz, additional, and Juarez, Gema Maria Fernandez, additional

Published
2024
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17. IgA Nephropathy Is the Most Common Underlying Disease in Patients With Anticoagulant-Related Nephropathy

Author

Trujillo, Hernando, Sandino, Justo, Cavero, Teresa, Caravaca-Fontán, Fernando, Gutiérrez, Eduardo, Sevillano, Ángel M., Shabaka, Amir, Fernández-Juárez, Gema, Rodríguez Doyágüez, Pablo, Gimena Muñoz, Rocío, Calle García, Leonardo, Cabello, Virginia, Muñoz-Terol, José Manuel, García Santiago, Ana, Toldos, Oscar, Moreno, Juan Antonio, and Praga, Manuel

Published
2022
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18. Cystic phenotype and chronic kidney disease in autosomal dominant Alport syndrome.

Author

Bada-Bosch, Teresa, Sevillano, Angel M, Sánchez-Calvin, María Teresa, Palma-Milla, Carmen, Cáceres, Ignacio Alba de, Díaz-Crespo, Francisco, Trujillo, Hernando, Alonso, Marina, Cases-Corona, Clara, Shabaka, Amir, Quesada-Espinosa, Juan Francisco, Lezana-Rosales, José Miguel, Gutiérrez, Eduardo, Fernández-Juárez, Gema, Caravaca-Fontán, Fernando, and Praga, Manuel

Subjects
CYSTIC kidney disease, CHRONIC kidney failure, KIDNEY diseases, GLOMERULAR filtration rate, BASAL lamina, FOCAL segmental glomerulosclerosis
Abstract

Background Autosomal dominant Alport Syndrome (ADAS), also known as thin basement membrane disease (TBMD), is caused by pathogenic variants in the COL4A3 and COL4A4 genes. A cystic phenotype has been described in some patients with TBMD, but no genetic studies have been performed. We conducted a genetic and radiologic investigation in a cohort of ADAS patients to analyze the prevalence of multicystic kidney disease (MKD) and its association with chronic kidney disease (CKD). Methods This was a retrospective single-center cohort study. Thirty-one patients showing pathogenic or likely pathogenic variants in COL4A3 or COL4A4 from a cohort of 79 patients with persistent microscopic hematuria were included. Mean follow-up was 9.4 ± 9.6 years. The primary objective of the study was to determine the prevalence of MKD in the cohort of ADAS patients. Secondary objectives were to determine risk factors associated with an estimated glomerular filtration rate (eGFR) <45 mL/min/1.73 m2 at the time of genetic and radiologic evaluation and to investigate the coexistence of other genetic abnormalities associated with familial hematuria and cystic kidney disease. Results MKD was found in 16 patients (52%). Mean number of cysts per kidney was 12.7 ± 5.5. No genetic abnormalities were found in a panel of 101 other genes related to familial hematuria, focal segmental glomerulosclerosis and cystic kidney disease. A greater number of patients with MKD had an eGFR <45 mL/min/1.73 m2 (63% vs 7%, P  = .006) and more advanced CKD than patients without MKD. The annual rate of eGFR decline was greater in patients with MKD: –1.8 vs 0.06 mL/min/1.73 m2/year (P  = .009). By multivariable linear regression analysis, the main determinants of eGFR change per year were time-averaged proteinuria (P  = .002) and MKD (P  = .02). Conclusion MKD is commonly found in ADAS and is associated with a worse kidney outcome. No pathogenic variants were found in genes other than COL4A3 / COL4A4. [ABSTRACT FROM AUTHOR]

Published
2024
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21. The STARMEN trial indicates that alternating treatment with corticosteroids and cyclophosphamide is superior to sequential treatment with tacrolimus and rituximab in primary membranous nephropathy

Author

Praga, Manuel, Sevillano, Angel, Caravaca-Fontan, Fernando, Trujillo, Hernando, Gutiérrez, Eduardo, Juarez, Gema Fernandez, Shabaka, Amir, Rojas-Rivera, Jorge, Egido, Jesus, Ortiz, Alberto, Goicoechea, Marian, Verdalles, Úrsula, Segarra, Alfons, Agraz, Irene, Martín-Reyes, Guillermo, Perea, Lara, Valera, Ildefonso, Martín, Mónica, Cabello, Virginia, Pérez Valdivia, Miguel Angel, Quintana, Luis F., Blasco, Miquel, Cao, Mercedes, Muñiz, Andrés López, Avila, Ana, Malek, Tamara, Espinosa, Mario, Rabasco, Cristina, Diaz, Montserrat, DaSilva, Iara, Bonet, Jordi, Navarro, Maruja, Gómez-Martino, Juan Ramón, Rivas, Begoña, Rodríguez-Moreno, Antolina, Galeano, Cristina, Romera, Ana, Huerta, Ana, Rodríguez-Paternina, Ezequiel, Vigil, Ana, Alcázar, Roberto, Paraíso, Vicente, Barrio, Vicente, Wetzels, Jack, Logt, Anne-Els van de, Hofstra, Julia, Fernández-Juárez, Gema, Justino, Joana, Caravaca-Fontán, Fernando, Ávila, Ana, Varela, Alfonso, Díez, Montserrat, Diezhandino, Marian Goicoechea, Bonet, Jose, Plaisier, Emmanuelle, Segarra, Alfonso, Lambeau, Gérard, and Ronco, Pierre

Published
2021
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23. Sodium-glucose cotransporter 2 inhibition in primary and secondary glomerulonephritis.

Author

Caravaca-Fontán, Fernando, Stevens, Kate, Padrón, Maite, Huerta, Ana, Montomoli, Marco, Villa, Juan, González, Fayna, Vega, Cristina, Mendoza, Manuel López, Fernández, Loreto, Shabaka, Amir, Rodríguez-Moreno, Antolina, Martín-Gómez, Adoración, Labrador, Pedro J, Andújar, Alicia Molina, Soler, M Carmen Prados, Martín-Penagos, Luis, Yerovi, Estefanía, Zahonero, Laura Medina, and Flor, José Carlos De La

Subjects
GLUCOSE transporters, SODIUM-glucose cotransporters, SODIUM-glucose cotransporter 2 inhibitors, NEPHRITIS, GLOMERULAR filtration rate, GLOMERULONEPHRITIS, BODY mass index, KIDNEY glomerulus diseases
Abstract

Background The role of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in the management glomerular/systemic autoimmune diseases with proteinuria in real-world clinical settings is unclear. Methods This is a retrospective, observational, international cohort study. Adult patients with biopsy-proven glomerular diseases were included. The main outcome was the percentage reduction in 24-h proteinuria from SGLT2i initiation to 3, 6, 9 and 12 months. Secondary outcomes included percentage change in estimated glomerular filtration rate (eGFR), proteinuria reduction by type of disease and reduction of proteinuria ≥30% from SGLT2i initiation. Results Four-hundred and ninety-three patients with a median age of 55 years and background therapy with renin–angiotensin system blockers were included. Proteinuria from baseline changed by –35%, –41%, –45% and –48% at 3, 6, 9 and 12 months after SGLT2i initiation, while eGFR changed by –6%, –3%, –8% and –10.5% at 3, 6, 9 and 12 months, respectively. Results were similar irrespective of the underlying disease. A correlation was found between body mass index (BMI) and percentage proteinuria reduction at last follow-up. By mixed-effects logistic regression model, serum albumin at SGLT2i initiation emerged as a predictor of ≥30% proteinuria reduction (odds ratio for albumin <3.5 g/dL, 0.53; 95% CI 0.30–0.91; P  = .02). A slower eGFR decline was observed in patients achieving a ≥30% proteinuria reduction: –3.7 versus –5.3 mL/min/1.73 m2/year (P  = .001). The overall tolerance to SGLT2i was good. Conclusions The use of SGLT2i was associated with a significant reduction of proteinuria. This percentage change is greater in patients with higher BMI. Higher serum albumin at SGLT2i onset is associated with higher probability of achieving a ≥30% proteinuria reduction. [ABSTRACT FROM AUTHOR]

Published
2024
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26. Clinical profiles and patterns of kidney disease progression in C3 glomerulopathy

Author

Caravaca-Fontán, Fernando, Cavero, Teresa, Díaz-Encarnación, Montserrat M., Cabello-Chaves, Virginia, Ariceta, Gema, Quintana, Luis F., Marco, Helena, Barros, Xoana, Ramos, Natalia, Rodríguez-Mendiola, Nuria, Cruz, Sonia, Fernández-Juárez, Gema, Rodríguez, Adela, Pérez de José, Ana, Rabasco, Cristina, Rodado, Raquel, Fernández, Loreto, Pérez Gómez, Vanessa, Ávila, Ana, Bravo, Luis, Espinosa, Natalia, Allende, Natalia, Sánchez de la Nieta, María Dolores, Rodríguez, Eva, Rivas, Begoña, Melgosa, Marta, Huerta, Ana, Miquel, Rosa, Mon, Carmen, Fraga, Gloria, de Lorenzo, Alberto, Draibe, Juliana, González, Fayna, Shabaka, Amir, López-Rubio, María Esperanza, Fenollosa, María Ángeles, Martín-Penagos, L., Da Silva, Iara, Alonso Titos, Juana, Rodríguez de Córdoba, Santiago, Goicoechea de Jorge, Elena, Praga, Manuel, Instituto de Salud Carlos III, European Commission, Red de Investigación Renal (España), Comunidad de Madrid, Ministerio de Economía y Competitividad (España), Caravaca-Fontán, Fernando, Cavero, Teresa, Díaz-Encarnación, Montserrat M., Ariceta, Gema, Quintana, Luis F., Barros, Xoana, Ramos, Natalia, Rodríguez-Mendiola, Nuria, Fernández-Juárez, Gema, Pérez de José, Ana, Pérez Gómez, Vanessa, Allende, Natalia, Sánchez de la Nieta, María Dolores, Rivas, Begoña, Melgosa, Marta, Huerta, Ana, Miquel, Rosa, Mon, Carmen, de Lorenzo, Alberto, Draibe, Juliana, González, Fayna, Shabaka, Amir, Martín-Penagos, L., Rodríguez de Córdoba, Santiago, Goicoechea de Jorge, Elena, and Praga, Manuel

Abstract

14 p.-4 fig.-3 tab., Background C3 glomerulopathy is a rare kidney disease, which makes it difficult to collect large cohorts of patients to better understand its variability. The aims of this study were to describe the clinical profiles and patterns of progression of kidney disease., Methods This was a retrospective, observational cohort study. Patients diagnosed with C3 glomerulopathy between 1995 and 2020 were enrolled. Study population was divided into clinical profiles by combining the following predictors: eGFR under/above 30 ml/min per 1.73 m2, proteinuria under/above 3.5 g/d, and histologic chronicity score under/above 4. The change in eGFR and proteinuria over time was evaluated in a subgroup with consecutive measurements of eGFR and proteinuria., Results One hundred and fifteen patients with a median age of 30 years (interquartile range 19–50) were included. Patients were divided into eight clinical profiles. Kidney survival was significantly higher in patients with a chronicity score, Conclusions Kidney survival is significantly higher in patients with a chronicity score, Work in this study was supported by the Instituto de Salud Carlos III/Fondo Europeo de Desarrollo Regional (ISCIII/FEDER) grant PI16/01685 and PI19/1624, and Red de Investigación Renal (RedInRen) (RD12/0021/0029) (to MP), the Autonomous Region of Madrid (S2017/BMD-3673) (to MP). SRdeC is supported by Ministerio de Economia y Competitividad grant PID2019-104912RB-I00 y Autonomous Region of Madrid grant S2017/BMD-3673.

Published
2023

28. #5351 IMPACT OF ADD-ON STEROID AND IMMUNOSUPPRESSIVE TREATMENT IN PATIENTS WITH RAPIDLY PROGRESSIVE SECONDARY IGA NEPHROPATHY

Author

Shabaka, Amir, primary, Barceló, Sara Aldana, additional, Sevillano, Angel, additional, Pérez, Javier Vian, additional, Hadad-Arrascue, Fernando, additional, Rodriguez-Moreno, Antolina, additional, Huerta, Ana, additional, Reguera, María José, additional, Lorente, Loreto Fernandez, additional, Carrillo, Isabel Galan, additional, Sierra, Milagros, additional, and Juarez, Gema Maria Fernandez, additional

Published
2023
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29. #4928 CLINICAL AND HISTOLOGICAL CHARACTERISTICS AND LONG-TERM PROGNOSIS OF LATE-ONSET LUPUS NEPHRITIS

Author

Shabaka, Amir, primary, Mendoza, Ana, additional, Rodriguez-Moreno, Antolina, additional, Pamplona, Irene Agraz, additional, Rabasco, Cristina, additional, Procaccini, Fabio, additional, Cuellar, Hernando Trujillo, additional, Malek, Tamara, additional, Hadad-Arrascue, Fernando, additional, Cobelo, Carmen, additional, Gueorguieva, Antonia, additional, Sierra, Milagros, additional, Serrano, Mariana Garbiras, additional, and Juarez, Gema Maria Fernandez, additional

Published
2023
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30. Sequential Treatment with Tacrolimus and Rituximab vs. Alternating Corticosteroids and Cyclophosphamide in Primary Membranous Nephropathy (PMN): FR-OR53

Author

Juarez, Gema Fernandez, Rojas-Rivera, Jorge E., van de Logt, Anne-Els, Justino, Joana, Sevillano, Angel M., Caravaca-Fontan, Fernando, Avila, Ana, Rabasco, Cristina, Cabello, Virginia, Goicoechea, Marian, Quintana, Luis F., Agraz, Irene, Romera segorbe, Ana M., Shabaka, Amir, Espinosa, Mario, Egido, Jesus, Lambeau, Gerard J., Ronco, Pierre M., and Praga, Manuel

Published
2020
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31. Genetic testing in focal segmental glomerulosclerosis: in whom and when?

Author

Tato, Ana María, Carrera, Noa, García-Murias, Maria, Shabaka, Amir, Ávila, Ana, Mora, María Teresa Mora, Rabasco, Cristina, Soto, Karina, Alvarez, Francisco Jose de la Prada, Fernández-Lorente, Loreto, Rodríguez-Moreno, Antolina, Huerta, Ana, Mon, Carmen, García-Carro, Clara, Cabrera, Fayna González, Navarro, Juan Antonio Martín, Romera, Ana, Gutiérrez, Eduardo, Villacorta, Javier, and Lorenzo, Alberto de

Subjects
FOCAL segmental glomerulosclerosis, GENETIC testing, GLOMERULAR filtration rate, BODY mass index, SERUM albumin, GENETIC variation, PEDIATRIC nephrology
Abstract

Background Genetic causes are increasingly recognized in patients with focal segmental glomerulosclerosis (FSGS), but it remains unclear which patients should undergo genetic study. Our objective was to determine the frequency and distribution of genetic variants in steroid-resistant nephrotic syndrome FSGS (SRNS-FSGS) and in FSGS of undetermined cause (FSGS-UC). Methods We performed targeted exome sequencing of 84 genes associated with glomerulopathy in patients with adult-onset SRNS-FSGS or FSGS-UC after ruling out secondary causes. Results Seventy-six patients met the study criteria; 24 presented with SRNS-FSGS and 52 with FSGS-UC. We detected FSGS-related disease-causing variants in 27/76 patients (35.5%). There were no differences between genetic and non-genetic causes in age, proteinuria, glomerular filtration rate, serum albumin, body mass index, hypertension, diabetes or family history. Hematuria was more prevalent among patients with genetic causes. We found 19 pathogenic variants in COL4A3–5 genes in 16 (29.3%) patients. NPHS2 mutations were identified in 6 (16.2%) patients. The remaining cases had variants affecting INF2, OCRL, ACTN4 genes or APOL1 high-risk alleles. FSGS-related genetic variants were more common in SRNS-FSGS than in FSGS-UC (41.7% vs 32.7%). Four SRNS-FSGS patients presented with NPHS2 disease-causing variants. COL4A variants were the most prevalent finding in FSGS-UC patients, with 12 patients carrying disease-causing variants in these genes. Conclusions FSGS-related variants were detected in a substantial number of patients with SRNS-FSGS or FSGS-UC, regardless of age of onset of disease or the patient's family history. In our experience, genetic testing should be performed in routine clinical practice for the diagnosis of this group of patients. [ABSTRACT FROM AUTHOR]

Published
2023
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36. Longitudinal change in proteinuria and kidney outcomes in C3 glomerulopathy

Author

Caravaca-Fontán, Fernando, Díaz-Encarnación, Montserrat M., Cabello, Virginia, Ariceta, Gema, Quintana, Luis F., Marco, Helena, Barros, Xoana, Ramos, Natalia, Rodríguez-Mendiola, Nuria, Cruz, Sonia, Fernández-Juárez, Gema, Rodríguez, Adela, Pérez de José, Ana, Rabasco, Cristina, Rodado, Raquel, Fernández, Loreto, Pérez Gómez, Vanessa, Ávila, Ana, Bravo, Luis, Espinosa, Natalia, Allende, Natalia, Sánchez de la Nieta, María Dolores, Rodríguez, Eva, Olea, Teresa, Melgosa, Marta, Huerta, Ana, Miquel, Rosa, Mon, Carmen, Fraga, Gloria, de Lorenzo, Alberto, Draibe, Juliana, Cano-Megías, Marta, González, Fayna, Shabaka, Amir, López-Rubio, María Esperanza, Fenollosa, María Ángeles, Martín-Penagos, L., Da Silva, Iara, Alonso Titos, Juana, Rodríguez de Córdoba, Santiago, Goicoechea de Jorge, Elena, Praga, Manuel, Spanish Group for the Study of Glomerular Diseases (GLOSEN), Instituto de Salud Carlos III, European Commission, Red Española de Investigación Renal, Comunidad de Madrid, Ministerio de Ciencia, Innovación y Universidades (España), Ministerio de Economía y Competitividad (España), Caravaca-Fontán, Fernando [0000-0002-5830-9663], Díaz-Encarnación, Montserrat M. [0000-0001-5172-3370], Cabello, Virginia [0000-0002-0877-5498], Ariceta, Gema [0000-0003-1763-1098], Quintana, Luis F. [0000-0001-7582-8476], Barros, Xoana [0000-0001-9690-9769], Ramos, Natalia [0000-0001-9832-326X], Rodríguez-Mendiola, Nuria [0000-0001-6994-7161], Fernández-Juárez, Gema [0000-0001-6641-7763], Pérez de José, Ana [0000-0002-6952-1459], Pérez Gómez, Vanessa [0000-0003-4558-5236], Sánchez de la Nieta, María Dolores [0000-0001-8574-0013], Olea, Teresa [0000-0003-2370-1048], Melgosa, Marta [0000-0001-6236-414X], Huerta, Ana [0000-0003-3342-7628], de Lorenzo, Alberto [0000-0001-8847-083X], Draibe, Juliana [0000-0002-2819-8560], González, Fayna [0000-0002-2313-2511], Shabaka, Amir [0000-0001-7039-4701], Martín-Penagos, L. [0000-0003-0159-7358], Rodríguez de Córdoba, Santiago [0000-0001-6401-1874], Goicoechea de Jorge, Elena [0000-0002-4978-2483], Praga, Manuel [0000-0001-9270-1071], Caravaca-Fontán, Fernando, Díaz-Encarnación, Montserrat M., Cabello, Virginia, Ariceta, Gema, Quintana, Luis F., Barros, Xoana, Ramos, Natalia, Rodríguez-Mendiola, Nuria, Fernández-Juárez, Gema, Pérez de José, Ana, Pérez Gómez, Vanessa, Sánchez de la Nieta, María Dolores, Olea, Teresa, Melgosa, Marta, Huerta, Ana, de Lorenzo, Alberto, Draibe, Juliana, González, Fayna, Shabaka, Amir, Martín-Penagos, L., Rodríguez de Córdoba, Santiago, Goicoechea de Jorge, Elena, and Praga, Manuel

Subjects
Nephrology, Adult, medicine.medical_specialty, Adolescent, Glomerulonephritis, Membranoproliferative, 030232 urology & nephrology, Kidney failure, 030204 cardiovascular system & hematology, Lower risk, Kidney, 03 medical and health sciences, Joint models, Young Adult, 0302 clinical medicine, Glomerulonephritis, Glomerulopathy, Internal medicine, medicine, Humans, C3 glomerulopathy, Retrospective Studies, Transplantation, Proteinuria, business.industry, Proportional hazards model, urogenital system, Complement C3, medicine.disease, medicine.anatomical_structure, Kidney Failure, Chronic, medicine.symptom, business, Cohort study
Abstract

11 p.-4 fig.-4 tab., Introduction: The association between a change in proteinuria over time and its impact in kidney prognosis has not been analyzed in C3 glomerulopathy. This study aims to investigate the association between the longitudinal change in proteinuria and the risk of kidney failure., Methods: Retrospective, multicenter observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases (GLOSEN). Patients diagnosed with C3 glomerulopathy between 1995 and 2020 were enrolled. A joint modeling of linear mixed-effects models was applied to assess the underlying trajectory of a repeatedly measured proteinuria, and a Cox model to evaluate the association of this trajectory with the risk of kidney failure., Results: The study group consisted of 85 patients, 70 C3 glomerulonephritis and 15 dense deposit disease, with a median age of 26 years (range 13-41). During a median follow-up of 42 months, 25 patients reached kidney failure. The longitudinal change in proteinuria showed a strong association with the risk of this outcome, with a doubling of proteinuria levels resulting in a 2.5-fold increase of the risk. A second model showed that a ≥ 50% proteinuria reduction over time was significantly associated with a lower risk of kidney failure (HR: 0.79; 95% CI : 0.56-0.97; p < 0.001). This association was also found when the ≥50% proteinuria reduction was observed within the first 6 and 12 months of follow-up., Conclusion: The longitudinal change in proteinuria is strongly associated with the risk of kidney failure. The change in proteinuria over time can provide clinicians a dynamic prediction of kidney outcomes., This study was supported by the Instituto de Salud Carlos III/Fondo Europeo de Desarrollo Regional (ISCIII/FEDER) grant PI16/01685 and PI19/1624, and Red de Investigación Renal (RedInRen) (RD12/0021/0029) (to M.P.), the Autonomous Region of Madrid (S2017/BMD-3673) (to M.P.); E.G.d.J. was supported by the Spanish ‘Ministerio de Ciencia, Innovación y Universidades’ (RYC-2013-13395 and RTI2018-095955-B-100); S.R.d.C. was supported by Ministerio de Economía y Competitividad/FEDER grant SAF2015-66287R and Autonomous Region of Madrid grant S2017/BMD3673.

Published
2021

38. Kidney Biopsy in Patients with Cancer along the Last Decade: A Multicenter Study

Author

Bolufer, Mónica, primary, García-Carro, Clara, additional, Blasco, Miquel, additional, Quintana, Luis F., additional, Shabaka, Amir, additional, Rabasco, Cristina, additional, Draibe, Juliana, additional, Merino, Ana, additional, Melero, María Rosa, additional, Alonso, Fabiola, additional, Buxeda, Anna, additional, Batalha, Paula, additional, Visús, Maria Teresa, additional, and Soler, Maria José, additional

Published
2022
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42. MO052: New Trend in the Diagnosis of Kidney Disease in Cancer Patients: A Multicenter Study

Author

Bolufer, Mónica, primary, Garcia Carro, Clara, additional, Blasco, Miquel, additional, Fernando Quintana Porras, Luis, additional, Shabaka, Amir, additional, Rabasco, Cristina, additional, Draibe, Juliana, additional, Merino Ribas, Ana, additional, Rosa Melero Martin, Maria, additional, Alonso Garcia, Fabiola, additional, Buxeda, Anna, additional, Batalha, Paula, additional, Teresa Visus, Maria, additional, and José Soler Romeo, Maria, additional

Published
2022
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43. MO770: Are There Any Differences in Survival According to the Type of Tunnelled Central Venous Catheters?

Author

Maria Cases Corona, Clara, primary, Gruss, Enrique, additional, Cordon, Alfredo, additional, Furaz-Czerpak, Karina, additional, Patiño Gómez, Beatriz, additional, del Mar Andrés Vázquez, Maria, additional, Díaz Enamorado, Yunayka, additional, Dominguez Torres, Patricia, additional, Gallego Valcarce, Eduardo, additional, Shabaka, Amir, additional, and Maria Fernandez Juarez, Gema, additional

Published
2022
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44. Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy

Author

Caravaca-Fontán, Fernando, primary, Rivero, Marta, additional, Cavero, Teresa, additional, Díaz-Encarnación, Montserrat, additional, Cabello, Virginia, additional, Ariceta, Gema, additional, Quintana, Luis F, additional, Marco, Helena, additional, Barros, Xoana, additional, Ramos, Natalia, additional, Rodríguez-Mendiola, Nuria, additional, Cruz, Sonia, additional, Fernández-Juárez, Gema, additional, Rodríguez, Adela, additional, Pérez de José, Ana, additional, Rabasco, Cristina, additional, Rodado, Raquel, additional, Fernández, Loreto, additional, Pérez-Gómez, Vanessa, additional, Ávila, Ana, additional, Bravo, Luis, additional, Espinosa, Natalia, additional, Allende, Natalia, additional, Sanchez de la Nieta, Maria Dolores, additional, Rodríguez, Eva, additional, Olea, Teresa, additional, Melgosa, Marta, additional, Huerta, Ana, additional, Miquel, Rosa, additional, Mon, Carmen, additional, Fraga, Gloria, additional, de Lorenzo, Alberto, additional, Draibe, Juliana, additional, González, Fayna, additional, Shabaka, Amir, additional, López-Rubio, Maria Esperanza, additional, Fenollosa, María Ángeles, additional, Martín-Penagos, Luis, additional, Da Silva, Iara, additional, Alonso Titos, Juana, additional, Rodríguez de Córdoba, Santiago, additional, Goicoechea de Jorge, Elena, additional, and Praga, Manuel, additional

Published
2022
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45. Manejo de la inmunosupresión en pacientes trasplantados de riñón con COVID19. Estudio multicéntrico nacional derivado del registro COVID de la S.E.N

Author

López-Oliva, María O., primary, Pérez-Flores, Isabel, additional, Molina, María, additional, José Aladrén, Mª, additional, Trujillo, Hernando, additional, Redondo-Pachón, Dolores, additional, López, Verónica, additional, Facundo, Carme, additional, Villanego, Florentino, additional, Rodríguez, Marisa, additional, Carmen Ruiz, Mª, additional, Antón, Paula, additional, Rivas-Oural, Alba, additional, Cabello, Sheila, additional, Portolés, José, additional, de la Vara, Lourdes, additional, Tabernero, Guadalupe, additional, Valero, Rosalía, additional, Galeano, Cristina, additional, Moral, Esperanza, additional, Ventura, Ana, additional, Coca, Armando, additional, Muñoz, Miguel Ángel, additional, Hernández-Gallego, Román, additional, Shabaka, Amir, additional, Ledesma, Gabriel, additional, Martínez, Patricia, additional, Ángeles Rodríguez, Mª, additional, Tamajón, Lourdes Pérez, additional, Cruzado, Leónidas, additional, Emilio Sánchez, J., additional, and Jiménez, Carlos, additional

Published
2022
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47. Recurrent acute interstitial nephritis: what lies beneath

Author

Caravaca Fontán, Fernando, Shabaka, Amir, Sánchez Álamo, Beatriz, Lorenzo, Alberto de, Díaz, Martha, Blasco Pelícano, Miquel, Rodríguez, Eva, Sierra Carpio, Milagros, Malek Marín, Tamara, Urrestarazú, Andrés, Corona Cases, Clara, Praga, Manuel, Fernández Juárez, Gema, and Spanish Group for the Study of Glomerular Diseases (GLOSEN)

Subjects
Nephrology, medicine.medical_specialty, Interstitial nephritis, 030232 urology & nephrology, Tubulointerstitial nephritis and uveitis, recurrent acute interstitial nephritis, Azathioprine, 030204 cardiovascular system & hematology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, AcademicSubjects/MED00340, Transplantation, business.industry, Original Articles, medicine.disease, Etiology, acute interstitial nephritis, Sarcoidosis, business, chronic kidney disease, Kidney disease, Cohort study, medicine.drug
Abstract

Background Acute interstitial nephritis (AIN) is an emerging cause of acute kidney disease. While this disease usually follows an acute course, it may occasionally recur, representing a major challenge for the clinician. Methods We performed a retrospective, observational cohort study in 13 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. Patients with biopsy-proven AIN between 1996 and 2018 were included. Results The study group consisted of 205 patients with AIN, 22 of which developed recurrent AIN (RAIN) after a median of 111 days from diagnosis. RAIN was due to a surreptitious reintroduction of a previously known implicated drug or toxic in six patients (27%), sarcoidosis in two (9%), Sjögren’s syndrome in three (14%), light-chain-mediated AIN in two (9%) and tubulointerstitial nephritis and uveitis syndrome in two (9%), while in the rest of cases (32%), no precise cause could be identified. Microscopic haematuria was more frequent in patients with underlying systemic diseases. The first RAIN episode was treated with a repeated course of corticosteroids in 21 patients (95%). In six cases (27%), azathioprine and mycophenolate mofetil were added as corticosteroid-sparing agents. During a median follow-up of 30 months, 50 patients (27%) with no recurrences and 12 patients (55%) with RAIN reached Stages 4 and 5 chronic kidney disease (CKD). By multivariable logistic regression analysis, RAIN was independently associated with the risk of reaching Stages 4 and 5 CKD, even after adjusting for potential covariables. Conclusions RAIN is infrequent but is associated with poor kidney survival. RAIN should prompt clinicians to search for an underlying aetiology other than drug induced. However, in a large percentage of cases, no precise cause can be identified.

Published
2020

49. C3 glomerulopathy associated with monoclonal gammopathy: impact of chronic histologic lesions and beneficial effects of clone-targeted therapies

Author

Caravaca-Fontán, Fernando, primary, Lucientes, Laura, additional, Serra, Núria, additional, Cavero, Teresa, additional, Rodado, Raquel, additional, Ramos, Natalia, additional, Gonzalez, Fayna, additional, Shabaka, Amir, additional, Cabello, Virginia, additional, Huerta, Ana, additional, Pampa-Saico, Saúl, additional, Gutiérrez, Eduardo, additional, Quintana, Luis F, additional, López-Rubio, Maria Esperanza, additional, Draibe, Juliana, additional, Alonso Titos, Juana, additional, Fernández-Juárez, Gema, additional, Goicoechea de Jorge, Elena, additional, and Praga, Manuel, additional

Published
2021
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50. C3 glomerulopathy associated with monoclonal gammopathy: impact of chronic histologic lesions and beneficial effects of clone-targeted therapies.

Author

Caravaca-Fontán, Fernando, Lucientes, Laura, Serra, Núria, Cavero, Teresa, Rodado, Raquel, Ramos, Natalia, Gonzalez, Fayna, Shabaka, Amir, Cabello, Virginia, Huerta, Ana, Pampa-Saico, Saúl, Gutiérrez, Eduardo, Quintana, Luis F, López-Rubio, Maria Esperanza, Draibe, Juliana, Titos, Juana Alonso, Fernández-Juárez, Gema, Jorge, Elena Goicoechea de, and Praga, Manuel

Subjects
MONOCLONAL gammopathies, PROPENSITY score matching, KIDNEY failure, IMMUNOGLOBULIN G, TREATMENT effectiveness, RENAL biopsy, KIDNEY physiology
Abstract

Background C3 glomerulopathy associated with monoclonal gammopathy (C3G-MIg) is a rare entity. Herein we analysed the clinical and histologic features of a cohort of C3G-MIg patients. Methods We conducted a retrospective, multicentre, observational study. Patients diagnosed with C3G-MIg between 1995 and 2021 were enrolled. All had genetic studies of the alternative complement pathway. The degree of disease activity and chronicity were analysed using the C3G histologic index. Descriptive statistics and propensity score matching (PSM) analysis were used to evaluate the main outcome of the study [kidney failure (KF)]. Results The study group included 23 patients with a median age 63 of years [interquartile range (IQR) 48–70], and 57% were males. Immunoglobulin G kappa was the most frequent MIg (65%). The diagnosis of C3G-MIg was made in transplanted kidneys in seven patients (30%). Five (22%) patients had C3 nephritic factor and five (22%) had anti-factor H antibodies. One patient carried a pathogenic variant in the CFH gene. During a follow-up of 40 months (IQR 14–69), nine patients (39%) reached KF and these patients had a significantly higher total chronicity score on kidney biopsy. Patients who received clone-targeted therapy had a significantly higher survival compared with other management. Those who achieved haematological response had a significantly higher kidney survival. Outcome was remarkably poor in kidney transplant recipients, with five of them (71%) reaching KF. By PSM (adjusting for age, kidney function, proteinuria and chronicity score), no significant differences were observed in kidney survival between C3G patients with/without MIg. Conclusions The C3G histologic index can be used in patients with C3G-MIg to predict kidney prognosis, with higher chronicity scores being associated with worse outcomes. Clone-targeted therapies and the development of a haematological response are associated with better kidney prognosis. [ABSTRACT FROM AUTHOR]

Published
2022
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